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Sudden death and defibrillators in transposition of the great arteries with intra-atrial baffles: a multicenter study
Paul Khairy1, Louise Harris, Michael J Landzberg
1Canadian Adult Congenital Heart Network, Montreal, Quebec, Canada. paul.khairy@umontreal.ca
Insights
Implantable cardioverter defibrillators (ICDs) show high appropriate shock rates in secondary prevention for transposition of the great arteries patients. Beta-blockers may protect against shocks, while lead complications are frequent.
Area of Science:
- Cardiology
- Electrophysiology
- Congenital Heart Disease
Background:
- Transposition of the great arteries (TGA) with intra-atrial baffle repair carries a high risk of sudden cardiac death.
- Mechanisms of sudden death and the role of implantable cardioverter defibrillators (ICDs) in TGA patients are not well understood.
Purpose of the Study:
- To determine actuarial rates of ICD shocks in TGA patients.
- To identify risk factors for ICD shocks.
- To assess underlying arrhythmias and characterize complications associated with ICDs in TGA.
Main Methods:
- A multicenter cohort study involving 37 patients with TGA who received ICDs.
- Analysis of ICD shock rates, risk factors, arrhythmias, and complications.
- Primary vs. secondary prevention indications for ICD implantation were compared.
Main Results:
- Annual appropriate shock rates were 0.5% for primary and 6.0% for secondary prevention (P=0.0366).
- Independent predictors of appropriate shocks included secondary prevention indication and lack of beta-blocker use.
- Supraventricular tachycardia was implicated in 50% of appropriate shock episodes; inappropriate shocks and lead-related complications were common.
Conclusions:
- ICDs have high appropriate shock rates in secondary prevention for TGA patients, but not in primary prevention.
- Beta-blockers may offer protection against shocks, and inducible ventricular tachycardia did not predict future events.
- Inappropriate shocks and late lead-related complications are significant concerns in this population.
Background:
Transposition of the great arteries with intra-atrial baffle repair is among the congenital heart defects at highest risk of sudden death. Little is known about mechanisms of sudden death and the role of implantable cardioverter defibrillators.
Methods And Results:
We conducted a multicenter cohort study in patients with transposition of the great arteries to determine actuarial rates of implantable cardioverter defibrillator shocks, identify risk factors, assess underlying arrhythmias, and characterize complications. Overall, 37 patients (age, 28.0+/-7.6 years; 89.2% male) were enrolled from 7 sites. Implantable cardioverter defibrillators were implanted for primary prevention in 23 (62.1%) patients and secondary prevention in 14 patients (37.8%). Annual rates of appropriate shocks were 0.5% and 6.0% in primary and secondary prevention, respectively (P=0.0366). Independent predictors were a secondary prevention indication (hazard ratio, 18.0; P=0.0341) and lack of beta-blockers (hazard ratio, 16.7; P=0.0301). In patients with appropriate shocks, intracardiac electrograms documented supraventricular tachycardia preceding or coexisting with ventricular tachycardia in 50%. No patient with inducible ventricular tachycardia received an appropriate shock in comparison with 37.5% of noninducible patients (P=0.0429). Inappropriate shocks occurred in 6.6% per year, more so in patients of lesser weight (hazard ratio, 0.91 per kg; P=0.0168). Additionally, 14 patients (37.8%) experienced complications: 5 (13.5%) acute, 1 (2.7%) late generator related, and 12 (32.4%) late lead related.
Conclusions:
In patients with transposition of the great arteries, high rates of appropriate shocks are noted in secondary but not primary prevention. Supraventricular arrhythmias may be implicated in the etiology of ventricular tachyarrhythmias; beta-blockers seem protective, and inducible ventricular tachycardia does not seem to predict future events. Inappropriate shocks and late lead-related complications are common.
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